Bottom Line:
Diffuse plane xanthomas are characterized by the presence of yellowish plaques on the eyelids, neck, upper trunk, buttocks, and flexural folds.Approximately half of the cases are associated with lymphoproliferative disorders.Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of hepatic venous outflow.

Affiliation: Department of Dermatology, University of Kirikkale, School of Medicine, Ankara, Turkey. mukadderkocak@gmail.com

ABSTRACTDiffuse plane xanthomas are characterized by the presence of yellowish plaques on the eyelids, neck, upper trunk, buttocks, and flexural folds. Histology shows foamy histiocytes in the dermis. Approximately half of the cases are associated with lymphoproliferative disorders. Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of hepatic venous outflow. We present a case of diffuse plane xanthoma in a 62-year-old man who developed normolipemic plane xanthomas coinciding with Budd-Chiari syndrome and monoclonal gammopathy. We review the English-language literature regarding the rare association of xanthomas and Budd-Chiari syndrome.

Mentions:
Physical examination revealed flat, slightly infiltrated yellow-orange and yellow-brown plaques covering the forehead, eyelids, preauricular area, neck, proximal arms, upper trunk, buttocks, and lower extremity. Identical plaques were located in the lower and upper extremity in a more linear configuration [Figures 1 and 2] There were bruises over the plaques owing to severe and persistent itching [Figure 3]. Histological examination of skin biopsy revealed an infiltrate of foamy macrophages in the papillary dermis and perivascular region [Figure 4]. The foamy cells were negative for S-100 and CD1a antibodies. The overlying epidermis was normal. IgA, IgG, IgM, C3, and C4 were established to be negative in direct immunofluorescence investigations.

Mentions:
Physical examination revealed flat, slightly infiltrated yellow-orange and yellow-brown plaques covering the forehead, eyelids, preauricular area, neck, proximal arms, upper trunk, buttocks, and lower extremity. Identical plaques were located in the lower and upper extremity in a more linear configuration [Figures 1 and 2] There were bruises over the plaques owing to severe and persistent itching [Figure 3]. Histological examination of skin biopsy revealed an infiltrate of foamy macrophages in the papillary dermis and perivascular region [Figure 4]. The foamy cells were negative for S-100 and CD1a antibodies. The overlying epidermis was normal. IgA, IgG, IgM, C3, and C4 were established to be negative in direct immunofluorescence investigations.

Bottom Line:
Diffuse plane xanthomas are characterized by the presence of yellowish plaques on the eyelids, neck, upper trunk, buttocks, and flexural folds.Approximately half of the cases are associated with lymphoproliferative disorders.Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of hepatic venous outflow.

Affiliation:
Department of Dermatology, University of Kirikkale, School of Medicine, Ankara, Turkey. mukadderkocak@gmail.com

ABSTRACTDiffuse plane xanthomas are characterized by the presence of yellowish plaques on the eyelids, neck, upper trunk, buttocks, and flexural folds. Histology shows foamy histiocytes in the dermis. Approximately half of the cases are associated with lymphoproliferative disorders. Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of hepatic venous outflow. We present a case of diffuse plane xanthoma in a 62-year-old man who developed normolipemic plane xanthomas coinciding with Budd-Chiari syndrome and monoclonal gammopathy. We review the English-language literature regarding the rare association of xanthomas and Budd-Chiari syndrome.